The Treatment Strategies for Kawasaki Disease without Response to Intravenous Immunoglobulin (IVIG)

Abstract

Background: Kawasaki Disease (KD) is an acute vasculitis that occurs predominantly in children. The standard treatment is initial therapy with intravenous immunoglobulin (IVIG), but some patients develop resistance to IVIG, which increases their risk of developing coronary artery lesions. This review aims to analyze and summarize the causes and pathological mechanisms of IVIG resistance, as well as other treatment strategies. Results: Studies have found that IVIG resistance is mediated by multiple factors including genetic susceptibility, immune dysregulation, and changes in vascular endothelial cell characteristics, with persistently elevated pro-inflammatory cytokines as the core manifestation. Second-dose IVIG, glucocorticoids, and infliximab as second-line treatments each have their advantages and disadvantages. A second IVIG dose is currently the preferred treatment for IVIG non-responders in KD, while infliximab is more effective in controlling fever and coronary inflammation. Combination therapy may be a better option. However, existing predictive models remain limited, with obvious heterogeneity in clinical data. Conclusion: The pathogenesis of Kawasaki disease remains unclear. Current treatment options still carry risks and controversies. Further experimental research is needed to elucidate resistance mechanisms, construct predictive models, conduct heterogeneous experiments, and promote international collaboration to achieve breakthroughs in the treatment of this disease.